The General Clinical Research Center of Emory University has a long history of outstanding clinical research. The primary objective of this 5-Year renewal application is to provide the facilities and resources whereby both the existing and an increasing number of outstanding new basic science and clinical investigators can pursue important areas of clinical research. The specific aims and major areas of research proposed are outlined in 44 defined protocols involving 76 investigators. These include: 1) studies on chronic liver diseases and complications such as metabolic bone disease; studies of hepatic function and hemodynamics in liver disease, including the effects of liver transplantation and modulation of hemodynamics by a variety of pharmacologic agents; 2) studies of kidney disease, including the mechanisms by which nutritional intervention might retard progression to end-stage renal disease; 3) studies in endocrinology, including obesity; alterations in glucose and lactate metabolism; insulin resistance and diabetes; genetic regulation of insulin receptors; therapeutic trials with growth hormone; and nutritional regulation of somatomedin and somatomedin inhibitors; 4) studies in hematology/oncology, including investigation of platelet production and destruction; 5) studies in cardiology, comparing balloon angioplasty with bypass graft surgery for treatment of coronary artery disease; 6) studies of inherited disorders of mitochondrial function, including the mitochondrial myopathies, and molecular approaches to nuclear mutations such as the Emery-Dreifuss muscular dystrophy; and 7) studies of inborn errors of metabolism, especially the enzymatic and molecular mechanisms producing maple syrup urine disease, phenylketonuria, hemochromatosis and porphyria. Overall, these projects focus on important medical, surgical and pediatric health disorders for which new discoveries and treatments could have a major impact. To accomplish these aims, we offer an outstanding team of existing and new investigators, a wealth of patient material from the Emory University Affiliated Hospitals and Clinic, a tightly administered 12-bed clinical research facility with experienced research nurses, a core laboratory with expertise in metabolic balance studies, a nutrition/calorimetry team and metabolic kitchen, a newly developed and equipped molecular biology laboratory, a highly organized biostatistical support unit and CLINFO computer resource, and a University with a vital commitment to excellence in research.